Gastrointestinal Duplications
By P.S. Puligandla, L.T. Nguyen, D. St-Vil, H. Flageole, A.L. Bensoussan, V-H. Nguyen, and J-M. Laberge
Montreal, Quebec
Background/Purpose: The aim of this study was to review
the presentations of gastrointestinal duplication (GID) and to
assess the influence of prenatal diagnosis on treatment.
Methods: Retrospective review of all GID at 2 pediatric hos-
pitals from 1980-2002 was conducted.
Results: Seventy-three patients (M43:F30) were identified: 21
neonates, 28 infants (1 to 24 months), 15 children (1 to 10
years), 9 adolescents (11 years). GID location by frequency
was ileum (31.5%), ileocaecal valve (30.2%), duodenum
(9.6%), stomach (8.2%), jejunum (8.2%), colon (6.8%), and
rectum (5.5%). In neonates and infants, vomiting and disten-
sion were the most common presentations. Volvulus, caused
by a duplication, occurred in 23.8% of neonates and caused
the death of one neonate. Intussusception was identified in
10.9% of patients. In older children and adolescents, pain and
vomiting were the most common associations. Six of these
patients were being treated for Crohn’s disease, with the
diagnosis of duplication made at laparotomy. Eighteen pa-
tients had a prenatal diagnosis by ultrasound scan, with
77.2% of these asymptomatic after birth. Most prenatal diag-
noses occurred after 1991 (77.8%). When comparing an ear-
lier period (1980 to 1991; 29 patients) with the current (1992
to 2002; 44 patients), a greater proportion of the latter pa-
tients were asymptomatic (36.4 v 13.8%) and had a lower
incidence of complications (volvulus/intussusception).
Conclusions: GID can lead to life-threatening complications.
Prenatal diagnosis should lead to expeditious postnatal in-
vestigation and treatment before the onset of symptoms or
complications. GID in older children can mimic Crohn’s dis-
ease. Laparoscopy/laparotomy should be considered in pa-
tients with atypical Crohn’s disease or when the diagnosis of
an intraabdominal mass is unclear.
J Pediatr Surg 38:740-744. © 2003 Elsevier Inc. All rights
reserved.
INDEX WORDS: Gastrointestinal duplication, prenatal diag-
nosis, laparoscopy.
G
ASTROINTESTINAL duplications (GID) are un-
common congenital lesions that can occur any-
where from the mouth to the anus and have a reported
incidence of 1 in 4,500.
1
Despite a variety of early
descriptions, Ladd coined the term alimentary tract du-
plications in 1937, which more effectively described the
clinical and pathologic aspects of these lesions.
2
Their
etiology is of embryonic origin and several theories have
been proposed to explain their development. These in-
clude the split notocord syndrome,
3
defects in recanali-
zation,
4
and as remnants of embryonic diverticula.
5
How-
ever, none of these theories alone is able to explain the
full diversity of these lesions.
The presentation of GID can vary according to the age
of the patient as well as their location. Some GID may be
totally asymptomatic, identified on routine physical ex-
amination or during investigations for other problems.
With the increasing use of prenatal ultrasound scan,
many are now being identified in utero. This review was
undertaken to highlight the varied presentations of GID
and to assess the influence of prenatal diagnosis on their
management.
MATERIALS AND METHODS
From 1980 to 2002, all patient records with a diagnosis of gastro-
intestinal duplication were reviewed at 2 tertiary care pediatric hospi-
tals. Esophageal duplication cysts were excluded in this review. Pa-
tients were divided into 4 groups based on age: neonates, infants (30
days to 23 months), children (2 to 10 years), and adolescents (11 to 17
years). Data collection included information on patient demographics,
the location of GID, patient signs and symptoms, the investigations
used in the diagnosis, the treatment provided, and the pathologic
evaluation of the resected specimens. The influence of prenatal diag-
nosis on the management of GID also was evaluated. This was done by
assessing the frequency of prenatal diagnosis as well as the onset of
symptoms or complications (volvulus, intussusception) between pa-
tients diagnosed in an earlier era (1980 to 1991) to patients diagnosed
in the current era (1992 to 2002).
RESULTS
Seventy-three patients were identified in our review,
including 43 boys and 30 girls. There were 21 neonates,
28 infants, 15 children, and 9 adolescents. Sixty-seven
percent of patients were under 24 months of age. The
location and frequency of GID are listed in Table 1. The
From the Divisions of Pediatric Surgery and Pathology, The Mon-
treal Children’s Hospital, Montreal, Quebec, Canada, and the Division
of Pediatric Surgery, Hopital Ste Justine, Montreal, Quebec, Canada.
Presented at the 34th Annual Meeting of the Canadian Association
of Paediatric Surgeons Vancouver, British Columbia, Canada, Septem-
ber 19-22, 2002.
Address reprint requests to Dr Jean-Martin Laberge, Division of
Pediatric Surgery, The Montreal Children’s Hospital, McGill Univer-
sity Health Center, 2300 Tupper St, Room C1137, Montreal, Quebec,
Canada.
© 2003 Elsevier Inc. All rights reserved.
0022-3468/03/3805-0021$30.00/0
10.1016/S0022-3468(03)00041-1
740 Journal of Pediatric Surgery, Vol 38, No 5 (May), 2003: pp 740-744